The thalassaemia syndromes / D.J. Weatherall.

  • David Weatherall
Date:
1972
Catalogue details

Licence: Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0)

Credit: The thalassaemia syndromes / D.J. Weatherall. Source: Wellcome Collection.

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    28o THE THALASSAEMIA SYNDROMES therapy. Wasi (1971) has suggested that this compUcation may result from streptococcal infection ; this idea needs careful study in a large series of children. Another rare but serious complication of splenectomy in children with haemolytic anaemia is that of post-operative thrombocytosis and thrombo¬ embolism. Hirsch and Dacie (1966) have noted a reciprocal relationship between the packed cell volume and platelet count in patients who have undergone splenectomy, i.e. if the response is poor as judged by persistence of anaemia the platelet count tends to rise. In a few cases this is accom¬ panied by thromboembolic disease. Such was the case in a patient studied by the authors who developed migrating thrombophlebitis followed by generalised thromboembolic disease after splenectomy for haemoglobin H disease. Anticoagulant therapy and the use of a variety of agents for reducing platelet adhesiveness were no help and the patient died in right-sided heart failure secondary to multiple pulmonary emboli. It is difficult to design any rational therapeutic regime for these patients because it is not clear whether the thrombocytosis is the causative factor or whether it is part of a more general hypercoaguable state. CHELATING AGENTS The present position regarding the prevention of iron overload is very unsatisfactory. Since there are no drug regimes which will prevent absorp¬ tion of iron the only lines to follow are those of avoiding oral iron prepara¬ tions and taking the precautions mentioned in the section on transfusion therapy. It is too early to say whether the regular use of chelating agents will reduce the severity of this complication. In fact although potent chelating agents have been available for at least 5 years there is still little information available about their real value in thalassaemia. This is because it takes at least 5-10 years to assess any regime designed to reduce mortality from iron-overload and few physicians have been keen to examine critically a pro¬ longed regime which is expensive, painful and probably of marginal benefit. Of the chelating agents available desferrioxamine (Desferal; Ciba) and diethylenetriamine pentacetate (DTPA; Geigy) have been studied in the greatest detail. The chemical and pharmacological properties of desferrioxamine have been fully reviewed by Keberle (1964a). After intra¬ muscular injection the iron chelate is excreted in both the urine and bile. There is no doubt that iron can be removed in relatively large amounts with both agents once visceral siderosis is established. The amount of iron excreted seems to be related to the serum iron level and iron binding
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